(II-1.A.4d) 30-year-old patient with arterial pulmonary hypertension and ascending aortic aneurysm Natasza Herman, Paweł Prochownik, Urszula Gancarczyk, Monika Komar, Bartosz Sobień, Piotr Podolec

  EXPERTISE 1 “Development of the European Network in Orphan Cardiovascular Diseases” “Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”

Posted in 1. Pulmonary hypertension, 4. PAH associated with, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.1) 51-year old woman with idiopatic pulmonary arterial hypertension, atrial flutter and thrombus in right atrium Barbara Widlińska, Marcin Waligóra, Anna Tyrka, Monika Komar, Agnieszka Sarnecka, Mateusz Brózda, Paweł Prochownik, Urszula Gancarczyk, Natasza Herman, Piotr Podolec

  EXPERTISE 1   “Development of the European Network in Orphan Cardiovascular Diseases” “Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”

Posted in 1. Idiopathic PAH, 1. Pulmonary hypertension, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1B.1) 59-year-old woman with non-ischemic dilated cardiomyopathy and secondary pulmonary hypertension reactive to sildenafil Paweł Iwaszczuk, Monika Smaś-Suska, Marta Szuksztul, Magdalena Kaźnica-Wiatr, Lidia Tomkiewicz-Pająk, Piotr Musiałek, Maria Olszowska, Piotr Podolec

“Development of the European Network in Orphan Cardiovascular Diseases” “Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”

Posted in 1. Pulmonary hypertension, 1. Severe pulmonary hypertension due to left heart diseases, B. Severe forms of non-low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.4d) 71-year-old patient with patent ductus arteriosus P. Prochownik, A. Sarnecka MD, G. Kopeć MD, PhD, M. Komar MD, PhD, Prof. P. Podolec MD, PhD

  BRIEF COMMENTS EXPERT: Krzysztof Szymoński, MD Affiliation: Department of Cardiovascular Surgery and Transplantology, John Paul II Hospital, Krakow, Poland COMMENT Due to current ESC guidelines the patient qualifies for PDA closure. Tricuspid valvuloplasty may also be considered. EXPERT: Grzegorz Kopeć, MD, PhD Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow COMMENT Because there is no possibility of pulmonary hypertension targeted treatment the patient qualifies for PDA closure. Coexisting tricuspid regurgitation seems to be secondary to …

Posted in 1. Pulmonary hypertension, 4. PAH associated with, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1B1) Spectacular improvement in a 50 year old urgent heart transplant candidate after 3-month phosphodiesterase type 5 inhibitor (sildenafil) treatment Marta Szuksztul, Paweł Rubiś MD, PhD, Monika Dzieciuch-Rojek, Sylwia Wiśniowska-Śmiałek MD, Dawid Kudliński, Agata Leśniak-Sobelga MD, PhD, Magdalena Kostkiewicz MD, PhD, Prof. Piotr Podolec MD, PhD

  BRIEF COMMENTS EXPERT: Prof. Roland Hetzer, MD, PhD Affiliation: German Heart Instiute, Berlin, Germany COMMENT The patient responded well to the administered treatment (improvement of LVEF and gradual reduction of pulmonary hypertension) although the LV size didn’t change. Due to severe mitral regurgitation mitral valve repair should be considered, cardiosurgical consultation is required. The patient may need heart transplantation in the future. Furthermore CRT may be an option if such a procedure is technically feasible. EXPERT: Prof. Egle Ereminiene, …

Posted in 1. Pulmonary hypertension, 1. Severe pulmonary hypertension due to left heart diseases, B. Severe forms of non-low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.1) Idiopathic pulmonary artery hypertension

  BRIEF COMMENTS EXPERT: Prof. Henryk Siniawski MD, PhD, cardiac surgeon Affiliation: Deutsches Herzzentrum, Berlin, Germany COMMENT What was the initial function of the heart – in case of iPAH usually only lungs transplantation is performed, RV function often normalizes after lung-transplantation. Perhaps solitary lung transplantation would safe patient’s coronary arteries. EXPERT: Prof. Egle Ereminiene MD, PhD Affiliation: Hospital of Lithuanian University of Health Sciences, Kaunas COMMENT Poor RV function was the main reason of complex lungs and heart transplantation. …

Posted in 1. Idiopathic PAH, 1. Pulmonary hypertension, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.5) 41-year-old patient with chronic thromboembolic pulmonary hypertension Marcin Waligóra MD, Agnieszka Sarnecka MD, Grzegorz Kopeć MD, PhD, Prof. Piotr Podolec MD, PhD

https://i1.wp.com/www.crcd.eu/wp-content/uploads/2013/07/image11.png?fit=190%2C169

Background Acute pulmonary embolism is rarely complicated by development of pulmonary hypertension (PH) with estimated incidence rate of 0,5-2%. It is almost impossible to determine the overall prevalence of chronic thromboembolic pulmonary hypertension (CTEPH) since not all of these patients have a history of acute pulmonary embolism. For patients after acute pulmonary embolism, diagnosis can be made after 3 months of effective anticoagulation, and remaining signs of PH. Final diagnosis can be made after confirming pre-capillary PH during right heart …

Posted in 1. Pulmonary hypertension, 5. Chronic thromboembolic pulmonary hypertension, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.4b) Pulmonary arterial hypertension associated with HIV and HCV infection Michał Furdal, Ewa Mroczek, Jakub Stępniewski

Background Pulmonary arterial hypertension (PAH) is a rare, but one of the most frequent complications of HIV infection. 3- year survival of patients with HIV related PAH (HIV-PAH) remaining in functional class III or IV by WHO (FC WHO) reaches approximately 20-30%. It is believed that highly active antiretroviral therapy (cART) or dysfunction of immune system and CD4 lymphocytes has less likely influence on the occurrence or the course of PAH. There is still limited data on the utilization of …

Posted in 1. Pulmonary hypertension, 4. PAH associated with, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.5) A 39-year-old woman with atypical variant of Klippel-Trenaunay syndrome and progressive thromboembolic pulmonary hypertension, successfully treated by pulmonary thromboendarterectomy Poręba M, Misztal M, Biederman A, Skrzypek A, Szatkowski P, Pirsztuk E, Czernecka-Mroczek D, Gackowski A , Nessler J

Background Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular malformation of veins, capillaries and lymphatic vessels which can be associated with thromboembolic events. We report on a female with severely complicated course of the disease. Case presentation We present a case of 39-year-old female with Klippel-Trenaunay syndrome (KTS) complicated by deep vein thrombosis and chronic thromboembolic pulmonary hypertension (CTEPH). Our patient was operated in adolescence and severe varicosities of her lower limb were excised. After second pregnancy she developed pulmonary …

Posted in 1. Pulmonary hypertension, 5. Chronic thromboembolic pulmonary hypertension, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment

(II-1A.4d) 47 years old female with Patent Ductus Arteriosus and Pulmonary Hypertension. Jakub Stępniewski MD, Grzegorz Kopeć MD, PhD, Hanna Dziedzic-Oleksy MD, Anna Tyrka MD, Prof. Piotr Podolec MD, PhD

Background Ductus arteriosus (DA) is a vascular connection between the main pulmonary artery and the aorta that allows blood to bypass the fetus’s non-functioning lungs. After delivery, the DA undergoes active constriction and obliteration eventually. A patent ductus arteriosus (PDA) occurs when the DA fails to close postnatally. PDA is an uncommon clinical finding in adult population. Improvement of survival of premature infants at risk for PDA and an increase in cases discovered incidentally, the incidence of PDA is rising …

Posted in 1. Pulmonary hypertension, 4. PAH associated with, A. Low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation | Leave a comment